Sood Akshay, Beckett William S, Cullen Mark R
Division of Pulmonary and Critical Care Medicine, Southern Illinois University School of Medicine, 701 North First Street, Room D434, PO Box 19636, Springfield, IL 62794-9636, USA.
Chest. 2004 Dec;126(6):2000-7. doi: 10.1378/chest.126.6.2000.
Chronic beryllium disease (CBD) shares many of its characteristics with sarcoidosis and is often treated with corticosteroids. There is limited available literature regarding the effect of long-term corticosteroid therapy on the natural history of CBD.
We conducted an observational retrospective study of six patients with CBD who received prolonged corticosteroid treatment with a mean pulmonary function test follow-up period of 10.1 years. Five of the six patients were exposed to beryllium at the same workplace. The diagnosis in four of the six cases was confirmed by a positive beryllium lymphocyte proliferation test result on blood or BAL fluid. Periodic pulmonary function tests were analyzed in relation to removal from beryllium exposure and treatment with corticosteroids.
Two broad patterns of response were noted in these patients. The first pattern seen in two patients showed no improvement in FVC or diffusion capacity of the lung for carbon monoxide (Dlco) with corticosteroids. However, a significant improvement in these parameters was noted on cessation of beryllium exposure in one of the two patients. The second pattern showed an initial improvement in FVC and Dlco with corticosteroids, which was not sustained. An improvement was noted on stopping beryllium exposure.
The response to long-term corticosteroids in CBD, quite like that in sarcoidosis, is variable. Significant lung function improvement may be seen following cessation of beryllium exposure.
慢性铍病（CBD）与结节病有许多共同特征，且常采用皮质类固醇进行治疗。关于长期皮质类固醇治疗对CBD自然病程影响的现有文献有限。
我们对6例接受长期皮质类固醇治疗的CBD患者进行了一项观察性回顾性研究，平均肺功能测试随访期为10.1年。6例患者中有5例在同一工作场所接触过铍。6例中有4例的诊断通过血液或支气管肺泡灌洗（BAL）液中铍淋巴细胞增殖试验结果呈阳性得以证实。对定期肺功能测试结果进行分析，以了解脱离铍接触及皮质类固醇治疗的情况。
在这些患者中观察到两种广泛的反应模式。在两名患者中看到的第一种模式显示，使用皮质类固醇时，用力肺活量（FVC）或肺一氧化碳弥散量（Dlco）没有改善。然而，在这两名患者中的一名停止接触铍后，这些参数有显著改善。第二种模式显示，使用皮质类固醇时FVC和Dlco最初有所改善，但未持续。停止接触铍后有改善。
CBD对长期皮质类固醇的反应，与结节病一样，是可变的。停止接触铍后，肺功能可能会有显著改善。
